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Spinocerebellar degeneration and slow saccades in three generations of a kinship: clinical and electrophysiologic findings

Estudo clínico e eletrofisiológico em três gerações de uma família com degeneração espinocerebelar e movimentos oculares lentos

Abstracts

Four members of a family with spinocerebellar degeneration and slow saccadic eye movements are described. Detailed electrophysiological studies revealed abnormalities of neurological pathways not apparent clinically. The patients had slow saccades as mesasured electrophysiologically, as well as absence of rapid eye movements (REM) despite REM stages of sleep. These studies suggest that although saccadic eye movement and REM are mediated through the pontine paramedian reticular formation, other characteristics of REM sleep are not necessarily mediated through the same neurons.


Este trabalho apresenta o estudo de quatro membros de uma família com degeneração espinocerebelar e movimentos sacádicos oculares lentos. Estudos neurofisiológicos detalhados mostraram anormalidades em tratos do sistema nervoso central que não eram aparentes ao exame clínico. Além dos movimentos oculares sacádicos lentos também foi possível demonstrar durante o registro poligráfico do sono a ausência completa dos movimentos oculares rápidos do sono REM apesar dos outros parâmetros do sono REM estarem normais. Desta maneira o estudo permite que se tire as seguintes conclusões: os movimentos sacádicos oculares lentos e os movimentos oculares rápidos do sono REM são mediados por mecanismo central comum possivelmente através da formação reticular paramediana da ponte; outras características fisiológicas do sono REM, particularmente a hipotonia muscular, não são necessariamente mediadas pelo mesmo sistema neuronal.


Spinocerebellar degeneration and slow saccades in three generations of a kinship: clinical and electrophysiologic findings

Estudo clínico e eletrofisiológico em três gerações de uma família com degeneração espinocerebelar e movimentos oculares lentos

Enaytolah Niakan; Tulio E. Bertorini; Helio Lemmi; Milton Medeiros; Richard Drewry; Edward Kish

From the Department of Neurology, and Department of Ophalmology, University of Tennessee Center for the Health Sciences; Neurodiagnostic Department and Sleep Disorders Center, Baptist Memorial Hospital; and St. Jude Children's Research Hospital; Memphis, Tennessee

SUMMARY

Four members of a family with spinocerebellar degeneration and slow saccadic eye movements are described. Detailed electrophysiological studies revealed abnormalities of neurological pathways not apparent clinically. The patients had slow saccades as mesasured electrophysiologically, as well as absence of rapid eye movements (REM) despite REM stages of sleep. These studies suggest that although saccadic eye movement and REM are mediated through the pontine paramedian reticular formation, other characteristics of REM sleep are not necessarily mediated through the same neurons.

RESUMO

Este trabalho apresenta o estudo de quatro membros de uma família com degeneração espinocerebelar e movimentos sacádicos oculares lentos. Estudos neurofisiológicos detalhados mostraram anormalidades em tratos do sistema nervoso central que não eram aparentes ao exame clínico. Além dos movimentos oculares sacádicos lentos também foi possível demonstrar durante o registro poligráfico do sono a ausência completa dos movimentos oculares rápidos do sono REM apesar dos outros parâmetros do sono REM estarem normais. Desta maneira o estudo permite que se tire as seguintes conclusões: os movimentos sacádicos oculares lentos e os movimentos oculares rápidos do sono REM são mediados por mecanismo central comum possivelmente através da formação reticular paramediana da ponte; outras características fisiológicas do sono REM, particularmente a hipotonia muscular, não são necessariamente mediadas pelo mesmo sistema neuronal.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Dr. E. Niakan was a recipient of a clinical fellowship from the Muscular Dystrophy Association of America.

Acknowledgements - We thank Dr. Alberto F. Heck, former Professor and Chairman, Department of Neurology. University of Tennessee Center for the Health Sciences and Mr. John Gilbert for their reviews and critiques of the manuscript; Sharon Burt and Joseph Belluomini for technical assistance; and Gayle Ownby and Becky Green for typing

Department of Neurology, The University of Tenessee Center for the Health Sciences - 800 Madison Avenue - Memphis, Tennessee 38163 - U.S.A.

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Publication Dates

  • Publication in this collection
    13 Aug 2012
  • Date of issue
    Sept 1984
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